Dorcet Guillaume, Benaiteau Marie, Ory-Magne Fabienne, Blancher Antoine, Pariente Jérémie, Fortenfant Françoise, Bost Chloé
Département de Neurologie, Hôpital Pierre Paul Riquet, CHU de Toulouse, Toulouse, France.
INSERM U1043 - CNRS UMR 5282, INFINITY, Toulouse, France.
Front Neurol. 2022 May 25;13:847798. doi: 10.3389/fneur.2022.847798. eCollection 2022.
Autoimmune encephalitis (AIE) is an increasingly broad nosological framework that may clinically mimic neurodegenerative diseases (NDDs).
We describe here the clinical, radiological, electrophysiological, and biological evolution of three patients. Two women aged 73 and 72 years and a 69-year-old man presented with complex cognitive and focal neurological symptoms and each had a predominant frontal dysexecutive involvement and an unexpectedly high titer of anti-MAG antibodies in the serum and cerebrospinal fluid (CSF). The question of an autoimmune cause was raised. After 2 years of follow-up and, for two of them, without improvement despite immunosuppressive treatments, diagnoses of NDD were eventually retained: post-radiation encephalopathy, progressive supranuclear palsy (PSP), and Alzheimer's disease.
The presence of a high titer of anti-MAG antibodies may be found in NDD. It could reflect cerebral tissue damages, particularly in the case of significant frontal involvement. Atypical presentations may lead to a search for a paraneoplastic neurologic syndrome or AIE. However, the indirect immunofluorescence staining positivity on a monkey cerebellum section linked with anti-MAG antibodies should not lead to those diagnoses being retained.
自身免疫性脑炎（AIE）是一个范围日益宽泛的疾病分类框架，在临床上可能类似于神经退行性疾病（NDDs）。
我们在此描述三名患者的临床、放射学、电生理学和生物学演变情况。两名分别为73岁和72岁的女性以及一名69岁男性出现复杂的认知和局灶性神经症状，且均以额叶执行功能障碍为主，血清和脑脊液（CSF）中抗MAG抗体滴度意外升高。于是提出了自身免疫病因的问题。经过2年的随访，其中两名患者尽管接受了免疫抑制治疗但仍无改善，最终确诊为NDD：放射性脑病、进行性核上性麻痹（PSP）和阿尔茨海默病。
在NDD中可能会发现高滴度抗MAG抗体。它可能反映脑组织损伤，特别是在额叶受累明显的情况下。非典型表现可能会促使寻找副肿瘤性神经综合征或AIE。然而，与抗MAG抗体相关的猴小脑切片间接免疫荧光染色阳性不应导致保留这些诊断。
